Jeffrey R. Avansino

Correlating Haller Index and cardiopulmonary disease in pectus excavatum.

BACKGROUNDnThe Haller Index (HI) has become standard for determining the severity of pectus excavatum. We compared patterns of cardiopulmonary dysfunction and their relationship with HI in patients with pectus excavatum.nnnMETHODSnWe performed cardiopulmonary exercise testing and chest computed tomography scans on 90 patients with pectus excavatum deformities at a regional pediatric hospital.nnnRESULTSnThe median HI was 4.9 in patients with combined dysfunction, 4.4 in patients with isolated pulmonary dysfunction, 3.6 in patients with isolated cardiac dysfunction, and 3.4 in patients with normal function. HI varied significantly by disease group (P < .009). HI was significantly lower in patients with normal forced vital capacity than with abnormal forced vital capacity (P = .001). However, HI was similar in patients with normal and abnormal oxygen pulse (P = .24) or peak oxygen consumption (P = .37).nnnCONCLUSIONSnFifty-nine percent of patients had cardiac and/or pulmonary limitation. A HI greater than 3.6 is associated with pulmonary dysfunction, but not cardiac dysfunction.

Implementation of a standardized procedural checklist in a children's hospital

BACKGROUNDnThe aim of this study was to examine the implementation and sustainability of checklist use among procedural-based specialties in a pediatric hospital and to survey perceptions of checklist efficacy among staff members and physicians.nnnMETHODSnA modified World Health Organization procedural checklist was implemented. Percentage daily compliance was collected for all procedures over a 12-month period. Clinical staff members participated in a survey regarding the checklist. Associations among gender, clinical role, and years of experience were evaluated.nnnRESULTSnCompliance at 12 months was significantly higher than at inception (94% vs 88%, P < .001) with average monthly compliance of 92%. Compliance deteriorated on weekends and holidays. Surgeons had more positive perceptions of using the checklist compared with nurses and anesthesiologists, independent of experience and gender (P = .001).nnnCONCLUSIONSnThe implementation of a procedural checklist can be sustained across specialties with high compliance. Off-hours utilization remains problematic. Perceptions of checklist efficacy are disparate among roles in the operating room.

Upper thoracic shape in children with pectus excavatum: Impact on lung function

Pectus excavatum (PE) can present with respiratory complaints in childhood. However severity of the PE, measured by the Pectus Severity Index (PSI), correlates only modestly with reduced vital capacity (VC). We hypothesized that another upper thoracic feature, a pectus gracilis (PG) or slender chest, co‐exists with PE, and impacts lung function.

Distal Rectal Skip-Segment Hirschsprung Disease and the Potential for False-Negative Diagnosis:

In skip-segment Hirschsprung disease (SS-HSCR), an aganglionic segment of bowel, which extends proximally from the distal rectum, is interrupted by a ganglionated “skip segment.” Skip segments are usually located far proximal to the rectum where they do not interfere with initial diagnosis, although the possibility of distal SS-HSCR should be considered during interpretation of intraoperative biopsies or patients with atypical postoperative courses. We report 2 cases of SS-HSCR with skip areas in the distal rectum, 1 of which led to a false-negative diagnosis by suction rectal biopsy. These 2 cases of SS-HSCR, along with others in the literature, highlight the point that ganglionic skip segments can confuse clinicians and lead to inadequate bowel resection, diagnostic delay, or a false-negative diagnosis. The pathogenesis of SS-HSCR is discussed in light of recent discoveries regarding transmesenteric migration of vagal neural crest cells and the role of sacral neural crest cells in hindgut neurodevelopment.

Optimizing value utilizing Toyota Kata methodology in a multidisciplinary clinic

INTRODUCTIONnValue in healthcare is measured in terms of patient outcomes achieved per dollar expended. Outcomes and cost must be measured at the patient level to optimize value. Multidisciplinary clinics have been shown to be effective in providing coordinated and comprehensive care with improved outcomes, yet tend to have higher cost than typical clinics. We sought to lower individual patient cost and optimize value in a pediatric multidisciplinary reconstructive pelvic medicine (RPM) clinic.nnnMATERIALS AND METHODSnThe RPM clinic is a multidisciplinary clinic that takes care of patients with anomalies of the pelvic organs. The specialties involved include Urology, General Surgery, Gynecology, and Gastroenterology/Motility. From May 2012 to November 2014 we performed time-driven activity-based costing (TDABC) analysis by measuring provider time for each step in the patient flow. Using observed time and the estimated hourly cost of each of the providers we calculated the final cost at the individual patient level, targeting clinic preparation. We utilized Toyota Kata methodology to enhance operational efficiency in an effort to optimize value. Variables measured included cost, time to perform a task, number of patients seen in clinic, percent value-added time (VAT) to patients (face to face time) and family experience scores (FES).nnnRESULTSnAt the beginning of the study period, clinic costs were

A painful protuberance

619 per patient. We reduced conference time from 6 min/patient to 1 min per patient, physician preparation time from 8 min to 6 min and increased Medical Assistant (MA) preparation time from 9.5 min to 20 min, achieving a cost reduction of 41% to

Use of 3D reconstruction cloacagrams and 3D printing in cloacal malformations

366 per patient. Continued improvements further reduced the MA preparation time to 14 min and the MD preparation time to 5 min with a further cost reduction to

Colocolonic intussusception in a four-yr-old with a heart transplant: A case report and review of the literature

194 (69%) (Figure). During this study period, we increased the number of appointments per clinic. We demonstrated sustained improvement in FES with regards to the families overall experience with their providers. Value added time was increased from 60% to 78% but this was not significant.nnnCONCLUSIONnTime-based cost analysis effectively measures individualized patient cost. We achieved a 69% reduction in clinic preparation costs. Despite this reduction in costs, we were able to maintain VAT and sustain improvements in family experience. In caring for complex patients, lean management methodology enables optimization of value in a multidisciplinary clinic.

Iatrogenic arteriovenous fistula in the Arm in an infant: Diagnostic and therapeutic considerations

s r h m a Case notes A 25-year-old woman with primary amenorrhea reported that a painful bulge intermittently appeared in her left groin. Her medical history also included known congenital left renal agenesis, tricuspid atresia, pulmonary stenosis, and a hypoplastic right ventricle that had required repair. Physical examination revealed Tanner V thelarche and adrenarche, a 1-cm vaginal dimple, and a left inguinal hernia. The patient’s karyotype was 46, XX. Pelvic ultrasound showed a right ovary. No left ovary, uterus, or vagina could be seen. Magnetic resonance imaging disclosed a right uterine remnant without functional endometrium and a normal right ovary. The left ovary appeared to be ectopically located; it was 5 cm below the inguinal ring, in the patent processus vaginalis (Figure 1). Diagnostic laparoscopy confirmed the presence of a normal right ovary and a small, nonobstructed, right uterine horn. The left ovary was adjacent to the internal inguinal ring, along with the source of the hernia: a chronically incarcerated left uterine remnant. The left uterine horn was partially reduced laparoscopically (Figure 2). The left round ligament, utero-ovarian ligament, and uterine vessels were isolated and ligated laparoscopically. The hernia sac was opened, and the left uterine remnant was mobilized and removed through the left herniorrhaphy incision. Pathology confirmed myometrial tissue without endometrial stroma or glands. The patient’s pain completely resolved.

The Pediatric Colorectal and Pelvic Learning Consortium (PCPLC): rationale, infrastructure, and initial steps

INTRODUCTIONnCloacal anomalies are complex to manage, and the anatomy affects prognosis and management. Assessment historically includes examination under anesthesia, and genitography is often performed, but these do not consistently capture three-dimensional (3D) detail or spatial relationships of the anatomic structures. Three-dimensional reconstruction cloacagrams can provide a high level of detail including channel measurements and the level of the cloaca (<3xa0cm vs. >3xa0cm), which typically determines the approach for surgical reconstruction and can impact long-term prognosis. Yet this imaging modality has not yet been directly compared with intra-operative or endoscopic findings.nnnOBJECTIVESnOur objective was to compare 3D reconstruction cloacagrams with endoscopic and intraoperative findings, as well as to describe the use of 3D printing to create models for surgical planning and education.nnnSTUDY DESIGNnAn IRB-approved retrospective review of all cloaca patients seen by our multi-disciplinary program from 2014 to 2016 was performed. All patients underwent examination under anesthesia, endoscopy, 3D reconstruction cloacagram, and subsequent reconstructive surgery at a later date. Patient characteristics, intraoperative details, and measurements from endoscopy and cloacagram were reviewed and compared. One of the 3D cloacagrams was reformatted for 3D printing to create a model for surgical planning.nnnRESULTSnFour patients were included for review, with the Figure illustrating 3D cloacagram results. Measurements of common channel length and urethral length were similar between modalities, particularly with confirming the level of cloaca. No patient experienced any complications or adverse effects from cloacagram or endoscopy. A model was successfully created from cloacagram images with the use of 3D printing technology.nnnDISCUSSIONnAccurate preoperative assessment for cloacal anomalies is important for counseling and surgical planning. Three-dimensional cloacagrams have been shown to yield a high level of anatomic detail. Here, cloacagram measurements are shown to correlate well with endoscopic and intraoperative findings with regards to level of cloaca and Müllerian development. Measurement discrepancies may be due to technical variation indicating a need for further evaluation. The translation of the cloacagram images into a 3D printed model demonstrates potential applications of these models for pre-operative planning and education of both families and trainees.nnnCONCLUSIONSnIn our series, 3D reconstruction cloacagrams yielded accurate measurements of urethral length and level of cloaca common channel and urethral length, similar to those found on endoscopy. Three-dimensional models can be printed from using cloacagram images, and may be useful for surgical planning and education.